2nsx

Structure of acid-beta-glucosidase with pharmacological chaperone provides insight into Gaucher disease
(see also Treatment of Gaucher disease)

 Superimposition of the native human acid β-glucosidase, expressed in cultured plant cells (pGlcCerase, 2v3f) on those of N-butyl-deoxynojirimycin/pGlcCerase (2v3d), N-nonyl-deoxynojirimycin/pGlcCerase (2v3e), and isofagomine/deglycosylated Cerezyme (IFG/DG-Cerezyme, 2nsx) reveals significant structural identity, neither of these ligands causes structural changes upon binding to the enzyme. The imino sugar of N-butyl-deoxynojirimycin (NB-DNJ) forms 7 hydrogen bonds and also makes several hydrophobic interactions with side chains of active site residues (2v3d). The crystal structure of pGlcCerase in complex with N-nonyl-deoxynojirimycin (NN-DNJ) (2v3e) is very similar to that of NB-DNJ /pGlcCerase. The exception is that longer chain of NN-DNJ interacts with 2 additional residues Leu241 (labeled lime ) and Leu314 of symmetrically related monomer (not shown). Comparison of the structures of NB-DNJ/pGlcCerase (2v3d) and NN-DNJ/pGlcCerase (2v3e) with that of <scene name='2v3d/Nsx/1'>IFG/DG-Cerezyme (2nsx) shows that the pyranose-like ring forms a same number of hydrogen bonds with the enzyme in all three cases (2v3d, 2v3e, and 2nsx).

Disease
Known disease associated with this structure: Gaucher disease, perinatal lethal OMIM:[606463], Gaucher disease, type I OMIM:[606463], Gaucher disease, type II OMIM:[606463], Gaucher disease, type III OMIM:[606463], Gaucher disease, type IIIC OMIM:[606463]

About this Structure
2NSX is a 4 chains structure of sequences from Homo sapiens. Full crystallographic information is available from OCA.